High rate of extreme thrombocytosis indicates bone marrow hyperactivity and splenic dysfunction among congenital diaphragmatic hernia patients

Abstract

Pediatric extreme thrombocytosis (EXT, platelet count > 1000 x 10³/µL) is rare. In a single center retrospective analysis of hospitalized children with EXT, infants with congenital diaphragmatic hernia (CDH) were overrepresented. In general pediatric patients, EXT is usually secondary to infection or inflammation, but most of the 14 CDH patients with EXT had no identifiable inciting factor. Instead, there was evidence that splenic dysfunction and bone marrow hyperactivity underlied EXT in CDH patients. None were associated with bleeding or thrombosis. Our findings identify mechanisms underlying EXT, and aid clinical interpretation and management of EXT in the pediatric population.

Keywords:

• Congenital diaphragmatic hernia
• NICU
• thrombocytosis

Acknowledgements

The authors thank the patients and families for whom we are privileged to care at the Children’s Hospital of Philadelphia.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Ethics statement

This study protocol was deemed exempt from oversight by the Children’s Hospital of Philadelphia Institutional Review Board.

Additional information

Funding

This work was funded through a grant from the National Institutes of Health (T32HD043021 to CST) and the Children’s Hospital of Philadelphia Division of Neonatology.